Thoracic manifestations of Behcet's syndrome.
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The author reports a case of Behcet's disease in a girl aged 18 years with recurrent lesions of the oral and vulvar mucosa, eyes and nervous system. The disease had a recurrent character with a tendency for progressing deterioration. Treatment was unsuccessful and the patient died.
The clinical response to levamisole in 11 patients with complete Behcet's syndrome was reviewed. Nine patients responded, 3 completely and 6 partially, with reduction in the number and severity of buccal and genital lesions. In 3 patients each, ocular inflammation and gastrointestinal involvement responded to levamisole, and in 1 patient neurologic status improved. Levamisole was purposely discontinued in 3 patients to assess its true role in disease control. Each patient experienced a flare. Reintroduction of therapy controlled the flare in all cases, although 1 patient subsequently relapsed while continuing therapy. Two patients failed to respond. Side effects necessitated permanent discontinuation of the drug in 2 respondent patients, but in no case did neutropenia or agranulocytosis develop. These preliminary results suggest that levamisole may be useful in the therapy of the various manifestations of Behcet's syndrome and that a controlled prospective study is indicated.
BACKGROUND: The pustular skin lesions of Behcet's syndrome (BS) are clinically and histopathologically similar to ordinary acne, but BS patients get lesions at sites not commonly involved in acne, such as the legs and arms. The microbiology of these lesions has not been studied adequately. OBJECTIVE: To make a detailed study of the microbiology of BS lesions. METHODS: Subjects were patients with BS and acne vulgaris. Material was extracted from pustular lesions and directly plated to aerobic and anaerobic media by sterile swab. Anaerobic bacteria were identified using a commercial kit (API 20A). Aerobic bacteria were defined by standard procedures. RESULTS: 58 BS patients and 37 acne patients were studied. Pustules were cultured from the following sites: BS patients (70 pustules): face (17), back (30), chest (2), arm (4), leg (17); acne patients (37 pustules): face (27), back (6), chest (1), arm (2), leg (1). At least one type of microorganism was grown from each pustule. Staphylococcus aureus (41/70, 58.6%, p = 0.008) and Prevotella spp (17/70, 24.3%, p = 0.002) were significantly more common in pustules from BS patients, and coagulase negative staphylococci (17/37, 45.9%, p = 0.007) in pustules from acne patients. CONCLUSIONS: The pustular lesions of BS are not usually sterile. The microbiology of these lesions is different from ordinary acne. It remains to be determined whether the infection is secondary or has any pathogenic implications.
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Serum IgG, IgM, IgA, IgD and IgE concentrations were examined in 26 patients with Behcet's syndrome, 70 patients with recurrent aphthous stomatitis and 56 healthy controls. IgA concentrations, but not IgG, IgM, IgD or IgE, were significantly raised in Behcet's syndrome compared with controls. Serum IgD and IgE concentrations but not IgA, IgG, or IgM were significantly greater in recurrent aphthous stomatitis than in controls.
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Is introducing a case study on a 20 year old female first time pregnant who presents with rare case of Adamantiadis-Bechet syndrome. These symptoms first appeared at the age of 9. The patient refers that from this age she has suffered from recurrent relapses of ulcerations of the mouth and genital organs, vescicular exanthema of the upper and lower extremities and severe headaches that would not subside with analgetic therapy. The patient upon the first trimester presented with a relapse of the disease and significant improvement during the remainder of the pregnancy. She delivered on the 39th week of her pregnancy a neonate male, alive and weighing 3640 gr. The Apgar--score 9 and 10 the 1st and 5th minute. Mother and neonate were discharged from the clinic on the 3rd postpartum day in good health. This case is also being discussed in international bibliography. Adamantiadis-Behcet syndrome constitutes systemic angiitis of unknown etiology that is characterized mainly by recurrent ulcers of the mouth and genital organs, serum negative arthritis, central nervous system disorders as well as ulcerations of the epidermis, nodular erythema, thrombophlebitis and vision disorders. It usually affects people in early adulthood, more often those in their 30s. Geographically it is seen more often in Japan, Korea, Turkey, Israel and other Mediterranean countries. Familial prevalence is also common.
Thirty-two patients with Behcet's syndrome were surveyed clinically, radiologically, and immunologically. HLA antigens were determined in a proportion. The patients were culled from a defined geographical area (Yorkshire) with a population of 5 million. 21 patients satisfied Mason and Barnes's (1969) criteria for major disease. 100% had mouth ulceration, 91% genital ulceration, 66% skin complaints, and 63% arthritis (of the nonerosive type previously documented). 3 patients had gastrointestinal involvement, one of whom had proven Crohn's disease. One patient had psoriasis. One patient had a popliteal artery aneurysm resected and one has since had surgery after the development of a tracheo-osophageal fistula. No sacroilititis was found.
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